Zanchetta María Belén, Robbiani Damián, Oliveri Beatriz, Giacoia Evangelina, Frigeri Adriana, Kallsbrum Silvia, Salerni Helena, Lucas Sabrina, Diaz Adriana, Perez Betiana, Pieroni Luisina, Arce Lange María Auxiliadora, Tormo Silvina, Kitaigrodsky Ariela, Galich Ana María
IDIM, Instituto de Investigaciones Metabólicas, Buenos Aires, Argentina.
Facultad de Medicina, Cátedra de Osteología, Universidad del Salvador, Buenos Aires, Argentina.
J Endocr Soc. 2020 Nov 28;5(2):bvaa181. doi: 10.1210/jendso/bvaa181. eCollection 2021 Feb 1.
Hypoparathyroidism is a rare disease and, as such, its natural history, long-term complications, and correct clinical management remain unclear.
To describe the natural history and clinical characteristics of the disease.
To present a retrospective observational analysis from 7 specialized centers in Buenos Aires, Argentina.
Chronic hypoparathyroid patients followed-up between 1985 and December 2018.
Data on demographics, etiology, clinical complications, biochemical parameters, dual-energy x-ray absorptiometry (DXA) values, and treatment doses were collected.
A total of 322 subjects with chronic hypoparathyroidism were included; 85.7% were female, the mean age was 55.2 ± 16.8 years, and the mean age at diagnosis was 43.8 ± 16.8 years. Prevalence of surgical hypoparathyroidism was 90.7%, with the most common causes being thyroid carcinoma and benign thyroid disease. A history of hypocalcemia requiring hospitalization was present in 25.7% of the whole group and in 4.3% of patients who had a history of seizures. Overall, 40.9% of our patients had reported at least 1 neuromuscular symptom. Renal insufficiency was present in 22.4% of our patients and was significantly associated with age ( < 0.0001). Hyperphosphatemia was present in 42% of patients. A history of severe hypocalcemia, paresthesias, tetany, ganglia calcifications, seizures, and cataracts was significantly higher in nonsurgical patients.
Although these patients were followed-up by experienced physicians, clinical management was heterogeneous and probably insufficient to assess all the potential complications of this chronic disease. Almost 70% of the study's group of patients met the experts' indications for considering the use of rhPTH 1-84. Being aware of this fact is the 1 step in improving our medical management of this disease in the future.
甲状旁腺功能减退症是一种罕见疾病，因此其自然病史、长期并发症及正确的临床管理仍不明确。
描述该疾病的自然病史和临床特征。
对阿根廷布宜诺斯艾利斯7个专科中心进行回顾性观察分析。
1985年至2018年12月期间接受随访的慢性甲状旁腺功能减退患者。
收集人口统计学、病因、临床并发症、生化参数、双能X线吸收测定（DXA）值及治疗剂量的数据。
共纳入322例慢性甲状旁腺功能减退患者；85.7%为女性，平均年龄为55.2±16.8岁，诊断时的平均年龄为43.8±16.8岁。手术性甲状旁腺功能减退症的患病率为90.7%，最常见的病因是甲状腺癌和良性甲状腺疾病。25.7%的患者有需要住院治疗的低钙血症病史，有癫痫发作史的患者中这一比例为4.3%。总体而言，40.9%的患者报告至少有1种神经肌肉症状。22.4%的患者存在肾功能不全，且与年龄显著相关（P<0.0001）。42%的患者存在高磷血症。非手术患者中严重低钙血症、感觉异常、手足搐搦、神经节钙化、癫痫发作及白内障的病史显著更多。
尽管这些患者由经验丰富的医生进行随访，但临床管理存在异质性，可能不足以评估这种慢性病的所有潜在并发症。近70%的研究患者组符合专家关于考虑使用重组人甲状旁腺激素1-84的指征。认识到这一事实是未来改善我们对该疾病医疗管理的第一步。
